SkIndia Quiz 42: Multiple Slow-growing Papulonodular Lesions Over the Nose.

A 67-year-old lady presented with multiple, slow-growing, pinkish, smooth surfaced, sessile, coalescing nodules of size 0.8 × 0.6 cm and few discrete papules measuring about 0.5 × 0.3 cm over the bridge of nose for 6 years [Figure 1]. The papulonodular lesions were firm, nontender, and without any attachment to the underlying structures. Family history was unremarkable. Excisional biopsy of the discrete papules and a core biopsy from a section of the coalesced nodular lesion was done and sent for microscopic examination.

Figure 1

Multiple coalescing nodules and discrete papules over the nose

Histopathology revealed islands of epithelial cells arranged in the upper dermis under low power view [Figure 2]. Each lobule was composed of peripheral palisading of dark basaloid cells and relatively paler cells in the center. The tumor islands were surrounded by a basement membrane such as hyaline eosinophilic structure, which is appreciated under higher magnification [Figure 3].

Figure 2

Photomicrograph showing islands of epithelial cells in the upper dermis (H and E, ×10)

Figure 3

Photomicrograph showing epithelial lobules containing inner cells with pale nuclei and peripheral cells with dark nuclei (H and E, ×40)

Answer

Multiple cylindromas.

Discussion

Cylindroma (turban tumor), first described by Ancell in 1842, is predominantly of apocrine origin, though eccrine features have been observed in recent studies.[1] It is usually seen in middle aged females with scalp being the most common site. Sporadic cylindromas present as solitary nodule, whereas dominantly inherited familial cases as in Brooke–Spiegler syndrome have multiple tumors.[2] The loss of heterozygosity in the CYLD gene on chromosome 16q has been suggested in familial cylindromatosis.[3]

Histologically, it is a dermal tumor without any attachment to the epidermis and composed of islands of epithelial cells arranged like pieces of a jigsaw puzzle, surrounded by a basement membrane-like glassy eosinophilic hyaline sheath.[4] Each island comprises peripherally located basaloid cells with small dark nuclei and scant cytoplasm in a palisading pattern and a central zone of large cells with pale nuclei. Ductal structures with intraluminal eosinophilic material may be seen. Malignant cylindromas show absence of jigsaw pattern, hyaline sheaths as well as peripheral palisading.[5]

Treatment strategy includes removal of the tumor by excision, radiofrequency ablation, electrocautery, cryotherapy, and lasers. Association with other adnexal tumors and malignant transformation is common with multiple cylindromas and close follow-up is necessary. Multiple cylindromas are more prevalent over scalp than on the nose. We present this case for the uncommonness of sporadic multiple cylindromas.

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.